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CASE REPORT article

Front. Immunol.
Sec. Autoimmune and Autoinflammatory Disorders : Autoimmune Disorders
Volume 16 - 2025 | doi: 10.3389/fimmu.2025.1532627

Castleman disease misdiagnosed as immunoglobulin G4-related disease: A case report

Provisionally accepted
Na Li Na Li Lifen Xu Lifen Xu Xiaoxia Liu Xiaoxia Liu Pengjia Wu Pengjia Wu Jun Liu Jun Liu Jiashun Zeng Jiashun Zeng *
  • Affiliated Hospital of Guizhou Medical University, Guiyang, Guizhou Province, China

The final, formatted version of the article will be published soon.

    Castleman disease (CD) and immunoglobulin G4-related disease (IgG4-RD) are rare systemic immune-mediated disorders that share overlapping clinical features, posing significant challenges in differential diagnosis. Here, we present a case of generalized lymphadenopathy initially misdiagnosed as IgG4-RD, which demonstrated a poor response to hormonal therapy. Subsequent pathological biopsy and immunohistochemical analysis ultimately confirmed the diagnosis of CD. This case underscores the limitations of relying solely on serum IgG4 levels as a diagnostic marker to distinguish CD from IgG4-RD. Comprehensive evaluation, including clinical presentation, organ involvement, serological and pathological findings, as well as therapeutic response, is essential to ensure accurate diagnosis and timely management.

    Keywords: Castleman disease, IgG4-related disease, Immune-mediated disorders, lymphadenopathy, Accurate Diagnosis

    Received: 22 Nov 2024; Accepted: 09 Jan 2025.

    Copyright: © 2025 Li, Xu, Liu, Wu, Liu and Zeng. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence: Jiashun Zeng, Affiliated Hospital of Guizhou Medical University, Guiyang, 550004, Guizhou Province, China

    Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.